Pediatric Cardiology Fellow Central Michigan University Children's Hospital of Michigan Detroit, Michigan, United States
Abstract:
Background: Some patients with univentricular cardiac physiology present late to care, delaying the traditional three-stage palliation aimed at balancing their pulmonary and systemic circulation. A portion of these patients may be candidates to undergo single-stage palliation with the premise that improving pulmonary blood flow and volume-unloading the single ventricle more rapidly will preserve its longevity. Additionally, in resource-limited areas where access to care and follow up may be poor, single-stage palliation may be preferable. However, excessive pulmonary blood flow may lead to postoperative complications such as effusion and lymphatic congestion. Furthermore, acute volume-unloading of the single ventricle and may lead to poorly tolerated or even fatal diastolic dysfunction.
Case: A 3 year-old female immigrant from Yemen was evaluated for cyanosis. She was diagnosed with congenital heart disease at birth, but was not palliated due to lack of resources. Examination revealed saturation of 72%, mild developmental delay, failure to thrive, digital clubbing, and a 4/6 systolic ejection murmur. Echocardiogram showed L-looped double inlet left ventricle, L-malposed great arteries, dysplastic pulmonary valve with severe stenosis (74mmHg gradient), no atrioventricular valve regurgitation, and widely patent bulboventricular foramen (Figure 1A). There was normal systemic and pulmonary venous drainage and dextrocardia with abdominal situs solitus. Cardiac catheterization showed adequately-sized, confluent pulmonary arteries (Figure 1B) with mean pressure of 12mmHg, transpulmonary gradient of 6mmHg, and pulmonary vascular resistance of 2.6 indexed Woods units. Four months after presentation, she underwent primary lateral tunnel Fontan palliation with a 4.5mm fenestration. Her postoperative course was uncomplicated; she was extubated in the operating room and discharged on postoperative day 5 with saturation of 85%. About one month later, she underwent pericardiocentesis for moderate global pericardial effusion. At her 3-month postoperative follow up evaluation, she was doing well clinically with saturation of 91% and satisfactory weight gain.
Discussion: We present a case of primary Fontan palliation of a 3 year-old female with complex single ventricle physiology. Postoperative outcome was favorable. Patients with single ventricle physiology and satisfactory hemodynamics should be considered for primary Fontan palliation. Satisfactory anatomical and hemodynamic characteristics include adequately-sized pulmonary arteries and low pulmonary vascular resistance, as was seen in this patient.
